Two sisters with Reed’s syndrome: treatment with pregabalin

Cutaneous leiomyomas, which originate in the arrector pili muscles of the skin are rarely seen benign cutaneous tumors. Sometimes familial cutaneous and uterine leiomyomatosis can occur together, an autosomal dominant genetic condition called Reed syndrome or familial leiomyomatosis cutis et uteri. This disorder can be accompanied by malignancies, particularly by renal carcinoma. In this paper, two sisters with Reed syndrome are presented in view of the rarity of the disorder and good response to pregabalin therapy

Two sisters with Reed’s syndrome: treatment with pregabalin

Cutaneous leiomyomas, which originate in the arrector pili muscles of the skin are rarely seen benign cutaneous tumors. Sometimes familial cutaneous and uterine leiomyomatosis can occur together, an autosomal dominant genetic condition called Reed syndrome or familial leiomyomatosis cutis et uteri. This disorder can be accompanied by malignancies, particularly by renal carcinoma. In this paper, two sisters with Reed syndrome are presented in view of the rarity of the disorder and good response to pregabalin therapy

Synchronous papillary urothelial carcinoma of the bladder and squamous cell carcinoma with sarcomatoid differentiation: A case report

The incidence rate of multiple primary tumors is 37% in all types of cancer. A patient diagnosed with primary cancer is 1.29 times more likely to develop an additional primary cancer when compared with the general population. Furthermore, in patients diagnosed with primary cancer, the possibility of a secondary malignancy in the same or different organ is increased. Following the identification of a secondary tumor, the risk of relapse or metastasis must be considered. The present study reports the case of a 76-year-old man who was admitted to Sakarya University Training and Research Hospital (Sakarya, Turkey) with swelling of the head, which had been apparent for 15 days. An excisional biopsy of the temporal region was performed and was used to diagnose the patient with synchronous squamous cell carcinoma with sarcomatoid differentiation of the scalp. The patient was referred to the Department of Plastic Surgery (Sakarya University Training and Research Hospital) for resection; however, he refused treatment and was subsequently discharged. To the best of our knowledge, this patient represents the first case of synchronous skin malignancy and urothelial carcinoma of the bladder to be reported in the literature

The relationship between histological prostatitis and lower urinary tract symptoms and sexual function

ABSTRACT This prospective analysis assessed the effect of histological prostatitis on lower urinary tract functions and sexual function. The patients were separated into two groups as histologically observed prostatitis (Group A) and no prostatitis (Group B) according to the biopsy outcomes. International prostate symptom score, international index of erectile function-5 scores, maximal and average flow rate, and residual urine volumes were compared statistically between groups. There was no significant difference (P>0.05) in baseline age (t=0.64), body mass index value (t=0.51), prostate volume (t=0.87), prostate-specific antigen levels (t=0.43), maximal (t=0.84) and average flow rate (t=0.59), and post-void residual urine volume (t=0.71). Mean international prostate symptom score in patients with prostatitis was numerically but not significantly higher than that in those without prostatitis (t=0.794, P=0.066). Mean international index of erectile function-5 score in the prostatitis group was significantly lower than that in those without prostatitis (t=1.854, P=0.013). Histological prostatitis notably affected sexual function of patients and may serve as a major risk factor for sexual dysfunction while having little effect on lower urinary tract symptoms

Appendix Neuroendocrine Tumor: Retrospective Analysis of 4026 Appendectomy Patients in a Single Center

Background/Aim. Appendix tumors are mostly incidentally identified in patients who were operated with the diagnosis of acute appendicitis. They are detected in approximately 1% of appendectomy specimens. Neuroendocrine tumors (NETs) account for over 50% of appendix neoplasms. NETs appearing in the appendix can cause carcinoid syndrome. In our study, we aimed to retrospectively examine the clinical features of patients who underwent appendectomy with the diagnosis of acute appendicitis and diagnosed with appendix NET in the postoperative period. Materials/Methods. The records of 4026 patients who were operated with the diagnosis of acute appendicitis between January 2008 and January 2020 at the Department of General Surgery at the Sakarya University Faculty of Medicine, were evaluated retrospectively. Clinical findings, demographic data, surgical findings, and results of the patients with appendix NET, as a result of histopathology, were examined in detail. Results. 16 of 4026 patients were reported as NET. Nine of the patients were male, and seven were female. The average age was 33 (19–49). Any of the patients had no signs and symptoms of carcinoid syndrome. All tumors were located at the tip of the appendix, and the mean tumor diameter was 0.85 cm (0.3–2.5 cm). As a result of pathology, one patient had mesoappendix and one patient had serosa invasion. Right hemicolectomy was applied to both patients. In other patients, meso, serosa, and lymphatic invasion were not detected. Tumor size was 2.5 cm in one of the patients, 1.5 cm in one, and 1.4 cm in the other, and the others were below 1 cm. In the postoperative follow-up, all the patients were discharged on average 2.71 (2–6 days) days without any complications. Conclusion. Appendix NETs are mostly asymptomatic and localized in a distal third of the appendix. Symptoms are mostly related to tumor size and distant metastases. Clinical behavior and prognosis can best be predicted by tumor size. Complementary hemicolectomy is recommended for tumors larger than 2 cm and tumors smaller than 1 to 2 cm, such as mesoappendix invasion, positive or uncertain surgical margin, high proliferative rate, and angioinvasion. For tumors whose diameter is less than 1 cm, simple appendectomy alone is sufficient

Assessment of Aprotinin Loaded Microemulsion Formulations for Parenteral Drug Delivery: Preparation, Characterization, in vitro Release and Cytotoxicity Studies

WOS: 000372334800004PubMed ID: 26306401The object of the current study was to prepare novel microemulsion formulations of aprotinin for parenteral delivery and to compare in vitro characteristics and release behaviour of different Technetium-99m (Tc-99m)-Aprotinin loaded microemulsion formulations. In addition, cytotoxicity of microemulsion formulation was evaluated with cell culture studies on human immortalized pancreatic duct epithelial-like cells. For this aim, firstly, pseudo-ternary phase diagrams were plotted to detect the formulation region and optimal microemulsions were characterized for their thermodynamic stability, conductivity, particle size, zeta potential, viscosity, pH and in vitro release properties. For in vitro release studies aprotinin was labelled with Tc-99m and labelling efficiency, radiochemical purity and stability of the radiolabeled complex were determined by several chromatography techniques. Radiolabeling efficiency of Tc-99m-Aprotinin was found over than 90% without any significant changes up to 6 hours after labelling at room temperature. After that, in vitro release studies of Tc-99m-Aprotinin loaded microemulsions were performed with two different methods; dissolution from diffusion cells and dialysis bags. Both methods showed that release rate of Tc-99m-Aprotinin from microemulsion could be controlled by microemulsion formulations. Drug release from the optimized microemulsion formulations was found lower compared to drug solution at the end of six hours. According to stability studies, the optimized formulation was found to be stable over a period of 12 months. Also, human immortalized pancreatic duct epithelial-like cells were used to evaluate the cytotoxicity of optimum formulation. Developed microemulsion did not reveal cytotoxicity. In conclusion the present study indicated that the M1-APT microemulsion is appropriate for intravenous application of aprotinin.Scientific and Technological Research Council of TurkeyTurkiye Bilimsel ve Teknolojik Arastirma Kurumu (TUBITAK) [Tubitak-108S083]This study was supported by The Scientific and Technological Research Council of Turkey (Tubitak-108S083). We would like to acknowledge Ege University Pharmaceutical Sciences Research Center (FABAL) for enabling us to use its laboratory instruments. The authors would like to thank to Ege University, Faculty of Pharmacy, and Department of Microbiology. The authors would like to thank Ismail Ozturk for assistance at sterility experiments

Differential expression of full-length and NH₂ terminally truncated FAM134B isoforms in normal physiology and cancer.

Selective autophagy of the endoplasmic reticulum (ER), namely ER-phagy, is mediated by ER-localized receptors, which are recognized and sequestered by GABARAP/LC3B-decorated phagophores and transferred to lysosomes for degradation. Being one such receptor, FAM134B plays critical roles in cellular processes such as protein quality control and neuronal survival. FAM134B has also been associated with different cancers, although its exact role remains elusive. We report here that the FAM134B gene encodes not one but at least two different protein isoforms: the full-length and the NH2 terminally truncated forms. Their relative expression shows extreme variation, both within normal tissues and among cancer types. Expression of full-length FAM134B is restricted to the brain, testis, spleen, and prostate. In contrast, NH2 terminally truncated FAM134B is dominant in the heart, skeletal muscle, kidney, pancreas, and liver. We compared wild-type and knockout mice to study the role of the Fam134b gene in starvation. NH2 terminally truncated FAM134B-2 was induced in the liver, skeletal muscle, and heart but not in the pancreas and stomach following starvation. Upon starvation, Fam134b(-/-) mice differed from wild-type mice by less weight loss and less hyperaminoacidemic and hypocalcemic response but increased levels of serum albumin, total serum proteins, and a-amylase. Interestingly, either NH2 terminally truncated FAM134B or both isoforms were downregulated in liver, lung, and colon cancers. In contrast, upregulation was observed in stomach and chromophobe kidney cancers